Aims To determine the incidence of retinoblastoma (Rb) and subsequent survival in the Finnish population during five decades.
Methods This retrospective observational cohort study comprised all patients with Rb born in Finland during 1964–2014 and diagnosed in 2018 (birth cohort analysis) or diagnosed in 1964–2014 (standard annual analysis), identified from the Finnish Cancer Registry and the national referral centre. We report age-adjusted incidences and survival according to cause of death.
Results Of children born in 1964–2014, 205 developed Rb, whereas 204 Rbs were diagnosed during these years; 196 belonged to both cohorts. Altogether 80 (38%) of the 213 children had heritable Rb and 19 (9%) had familial disease. The sex ratio was 1.34, suggesting male preponderance. Birth cohort analysis showed a median incidence of 6.2 per 100 000 live births (1:16 130) and less variability as compared with standard annual analysis (12.1, 6.5 and 4.4 per million children 0–4, 0–9 and 0–14 years of age, respectively). The incidence of heritable Rb increased with time, reflecting the increase in familial tumours. Five-year mortality rates from Rb were 6.2% and 7.6% for non-heritable and heritable diseases, respectively, and 35-year mortality rates from second malignancies were 0% and 14.3%, respectively. Family history predicted improved survival, whereas the period of diagnosis did not.
Conclusion The incidence of familial Rb has increased, along with improvement in survival in Finland in 1964–2014, whereas the overall incidence of Rb was stable. Long-term risk of dying of second malignancies after heritable Rb was in line with other countries.
- child health (paediatrics)
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Correction notice The funding statement has been updated since this article was published Online First.
Contributors KN conducted the work and TK was the supervisor. Both authors, KN and TK, contributed to the planning and reporting of the work, the design and implementation of the research, the analysis of the results and the writing of the manuscript.
Funding The authors received grants from two not-for-profit institutions: the Finnish Eye Foundation and the Finnish Eye and Tissue Bank Foundation.
Competing interests None declared.
Patient consent for publication Not required.
Ethics approval Our study was approved by the institutional review board of the Helsinki University Hospital and the National Institute for Health and Welfare. It followed the tenets of the Declaration of Helsinki.
Provenance and peer review Not commissioned; externally peer reviewed.
Data availability statement Data are available upon reasonable request. All data relevant to the study are included in the article or uploaded as supplementary information. All data relevant to the study are included in the article, due to patient and identity confideliality individual participant data is not shared. Additional enquiries on data are available upon reasonable request (firstname.lastname@example.org)
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